UNASSIGNED: The purpose of the study was to evaluate cochlea dimensions by the multiplanar reconstruction of high-resolution computed tomography that could be useful in diagnosing incomplete partition (IP) malformations.
UNASSIGNED: This study included 32 patients with 64 side cochleae diagnosed with IP defect and 38 cochleae as the control without any defect. Basal turn length (BL), cochlear height (CH), Mid-apical length (MAL), Mid-apical height, Cochlear length (A), and Cochlear width (B) were measured on reformat images.
UNASSIGNED: Twenty cochleae of these patients have been diagnosed with IP type I, 34 with IP type II, and 10 with IP type III. The MAL values are shorter than the control group in IP types I and III (p<0.001, p<0.001). BL values are shorter in IP type III cases (p<0.001). In IP II cases, BL and MAL values overlapped with the control group. CH did not differ significantly from the control group in any IP type. A and B values were significantly lower than the control group for IP I and III (p<0.01). There is a positive correlation between A and B values for all IP types (p<0.01).
UNASSIGNED: Quantitative data about differences in the size and shape of the cochlea in IP cases would help differentiate them from the normal cochlea. Since A and B values showed a positive correlation, it is suggested that A and B values can be used to estimate CDL for IP types.

OBJECTIVE: To share our experience of the surgical challenges faced in cochlear implanted patients with inner ear malformation and to assess the auditory and speech perception outcomes.
METHODS: Clinical records of 502 cochlear implant procedures were reviewed and data of 122 patients who had inner ear malformations were enrolled in the study. Their auditory and speech performances were evaluated post implantation for 3 years.
RESULTS: Cerebrospinal fluid gusher was encountered during opening of cochlea in 42 patients (34.4%) and one patient was re-explored within 24 h. In 30.3% of cases facial anomaly was found. Significant improvement in average performance was seen in all malformation types except in cochlear hypoplasia at 12 months postoperatively.
CONCLUSIONS: Surgical challenges can be overcome with expertise and giving special attention to preoperative imaging. Our experience suggests that outcomes are favourable in patients with inner ear malformations too.

OBJECTIVE: The present study aimed to evaluate binaural auditory skills in bimodal and bilateral pediatric cochlear implant (CI) users with incomplete partition type-II (IP-II) and to reveal the effect of IP-II on performance by comparing the results to pediatric CI users with normal cochlear morphology.
METHODS: Cross-sectional study.
METHODS: Tertiary referral center.
METHODS: Forty-one CI users (mean age 8.8 ± 1.9) were grouped as bimodal (BIM-IP) and bilateral (BIL-IP) users with IP-II; bimodal (BIM-N) and bilateral (BIL-N) users with normal cochlear anatomy. Speech perception in noise and sound localization skills were compared under 2 conditions; binaural (bilateral or bimodal) and monaural (first CI alone).
RESULTS: BIM-IP and BIL-IP showed no performance difference in binaural tasks. The BIM-N group showed remarkably poor performance in comparison to the groups of BIL-IP (p = .007), BIM-IP (p < .001), and BIL-N (p = .004) in terms of speech-in-noise skills. In sound localization abilities, similar significant differences were found between the group of BIM-N and the groups of BIL-IP (p = .001), BIM-IP (p < .001), and BIL-N (p = .004). All groups showed statistically significant improvements in binaural condition on both tasks (p < .05).
CONCLUSIONS: We revealed that bilateral and bimodal pediatric CI users with IP-II benefitted from implantation as much as bilateral users with normal anatomy. Differences in residual hearing between groups may explain the poor performance of bimodal users with normal cochlear morphology. To the best of our knowledge, it is the first study to unveil binaural performance characteristics in children diagnosed with a specific inner ear malformation subgroup.

BACKGROUND: High riding jugular bulb (HRJB) develops after 2 years and is rare at younger ages. High riding jugular bulb sometimes protrudes into the tympanic cavity, which can cause hemorrhagic complications during otologic surgery.
METHODS: We describe a congenitally deaf child with bilateral inner ear malformations and a right-sided HRJB on CT at 9 months. This child had undergone left cochlear implantation (CI) at 19 months, and right CI was planned at 6 years. However, we decided not to perform the right CI because preoperative CT images revealed that the right jugular bulb (JB) was enlarged and protruded into the tympanic cavity, completely covering the round window (RW).
CONCLUSIONS: This is the first pediatric case in which a longitudinal CT scan proves that HRJB develops and protrudes over time. These findings suggest that the earlier the HRJB forms, the larger it may grow. We must keep in mind the possibility of the enlargement and protrusion of HRJB.

Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.

BACKGROUND: Children with inner ear malformation (IEM) are at risk of vestibular loss as well as hearing loss. Incomplete partition (IP) anomalies constitute about 41% of all IEMs. This study aimed to investigate the postural control in subjects with the same type of IP on both sides and to compare their results with cochlear implant (CI) users without IEM and healthy peers.
METHODS: The study group consists of 17 subjects with the same IP types on both sides and using auditory implants on at least one side, with the following 3 groups: 6 IP-I subjects (mean age 12.28 ± 6.25), 6 IP-II subjects (mean age 12.90 ± 3.23), and 5 IP-III subjects (mean age 6.98 ± 3.10). Six unilateral CI users (mean age 11.38 ± 3.57) with normal inner ear structures were included in the CI control group, and 6 healthy peers (10.20 ± 4.79) were included in the healthy control group. The postural control was measured using the Bruininks-Oseretsky Test of Motor Proficiency Second Edition (BOT-2) balance subtest. All devices were turned off during the balance test.
RESULTS: The BOT-2 balance scale scores were observed to be significantly different between the IP-I and healthy control group (medians of balance scores being 3.00 and 16.00, respectively, p < 0.001) and the IP-III and healthy control group (medians of balance scores being 6.60 and 16.00, respectively, p = 0.04). The IP-II group had better balance scores (median = 8.00) than those of the other IP groups, although there were no significant differences between the IP-II and other groups (p > 0.05).
CONCLUSIONS: This study demonstrated that subjects with the same IP type on both sides and with early implantation may differ in terms of their postural control abilities depending on their IP type. Subjects with IP should be regularly followed up by the vestibular assessment and supported by their postural control ability by vestibular rehabilitation.

OBJECTIVE: To examine the outcomes of cochlear implantation in children with Pendred Syndrome.
METHODS: A retrospective case series of nine pediatric patients with Pendred syndrome undergoing cochlear implantation at a tertiary academic medical center from 2003 to 2017.
RESULTS: All patients were diagnosed with bilateral mild-to-profound to severe-to-profound sensorineural hearing loss and used hearing aids prior to implantation. Preoperative imaging results revealed that all patients exhibited bilateral enlarged vestibular aqueducts and 8 of 9 had cochlear dysplasia equivalent to Incomplete Partition II. Despite inner ear malformations, all electrodes were successfully implanted with minimal complications and favorable post-operative audiological outcomes.
CONCLUSIONS: Cochlear implantation is an effective and successful treatment for severe-to-profound hearing loss in children with Pendred syndrome and for whom traditional amplification aids provide limited benefit. With early intervention, cochlear implantation allows speech and cognitive development in Pendred children comparable to that of normal hearing individuals, allowing patients to learn in a mainstream educational setting.

Optimal matching of an electrode array to the cochlear anatomy plays a key role in bringing the best benefit of CI technology to the users. Even within the category of normal anatomy cochlea, the size variation is huge justifying MED-EL\'s FLEX electrode array to be available in five different lengths. Within the malformed inner-ear category the anatomical variation is huge, convincing MED-EL to custom-design the electrode array as per the request from the operating surgeons. Thanks to G. Bredberg, M. Beltrame, L. Sennaroglu, J. Gavilan, S. Plontke, T. Lenarz, J. Müller, and few others for their valuable suggestions on unique electrode designs satisfying various needs. Translational research efforts at MED-EL in cooperation with CI surgeons from across the world led to the implantation of a variety of electrode array designs in patients with special cochlear needs.

BACKGROUND: Certain inner ear malformations have stapes footplate fistula which may cause meningitis during otitis media. This may result in fatality. It is the responsibility of the otolaryngologist to diagnose and treat the condition to prevent further attacks of meningitis.
METHODS: Surgical findings of the 17 patients who have inner ear malformations with oval window fistula were retrospectively analyzed. Inner ear malformations were classified according to Sennaroglu classification. Different stages of stapes footplate fistula are classified.
RESULTS: Seventeen patients had spontaneous stapes footplate fistula at the oval window. No patient had spontaneous leakage at the round window site. Proper sealing of the leakage area with fascia in a dumbbell fashion is mandatory. Keeping the stapes in place and lumbar drainage are the two most important factors in the successful management of fistula. Particularly important is the simultaneous fistula repair and cochlear implantation where combined postauricular-transcanal approach provides the best method. Vaccination is important but not sufficient to prevent meningitis in inner ear malformations unless repair of the fistula is performed.
CONCLUSIONS: If the patient has a history of meningitis in the presence of inner ear malformation, particular attention should be given to oval window area to look for an opacity, cyst or a leaking lesion at the stapes footplate. Immediate surgical exploration and repair of the leak is mandatory to prevent further attacks of meningitis. Surgeon should not leave the operation without fully controlling the leak.

Cochleocele is an extrusion or herniation of the endosteum, through an incomplete stapes footplate, into the middle ear. The cochleocele may rupture resulting in a cerebrospinal fluid leak into the middle ear space causing a risk of menigitis. We report six cases of Incomplete Partition Type I with cochleocele which have all been successfully treated using a Totally Endoscopic Ear Surgery approach even during infancy. As the first two cases developed post-operative pseudomonas meningitis, preventative strategies are recommended.